Diencephalic Syndrome Due to Optic Pathway Gliomas in Pediatric Patients: An Italian Multicenter Study
Lucia De Martino,
Stefania Picariello,
Silvia Triarico,
Nicola Improda,
Pietro Spennato,
Michele Antonio Capozza,
Anna Grandone,
Claudia Santoro,
Daniela Cioffi,
Giorgio Attinà,
Giuseppe Cinalli,
Antonio Ruggiero,
Lucia Quaglietta
Affiliations
Lucia De Martino
Neurooncology Unit, Department of Pediatric Oncology, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Stefania Picariello
Neurooncology Unit, Department of Pediatric Oncology, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Silvia Triarico
Pediatric Oncology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 00168 Rome, Italy
Nicola Improda
Emergency Unit, Department of Emergency, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Pietro Spennato
Pediatric Neurosurgery Unit, Department of Pediatric Neurosciences, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Michele Antonio Capozza
Pediatric Oncology Unit, Department of Pediatric Oncology, Santobono-Pausilipon Children’s Hospital, via Posillipo n° 226, 80123 Naples, Italy
Anna Grandone
Department of Woman, Child and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, via L. De Crecchio n° 2, 80138 Naples, Italy
Claudia Santoro
Department of Woman, Child and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, via L. De Crecchio n° 2, 80138 Naples, Italy
Daniela Cioffi
Pediatric Endocrinology Unit, Department of Pediatrics, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Giorgio Attinà
Pediatric Oncology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 00168 Rome, Italy
Giuseppe Cinalli
Pediatric Neurosurgery Unit, Department of Pediatric Neurosciences, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Antonio Ruggiero
Pediatric Oncology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 00168 Rome, Italy
Lucia Quaglietta
Neurooncology Unit, Department of Pediatric Oncology, Santobono-Pausilipon Children’s Hospital, via M. Fiore n° 6, 80129 Naples, Italy
Diencephalic syndrome (DS) is a rare pediatric condition associated with optic pathway gliomas (OPGs). Since they are slow-growing tumors, their diagnosis might be delayed, with consequences on long-term outcomes. We present a multicenter case series of nine children with DS associated with OPG, with the aim of providing relevant details about mortality and long-term sequelae. We retrospectively identified nine children (6 M) with DS (median age 14 months, range 3–26 months). Four patients had NF1-related OPGs. Children with NF1 were significantly older than sporadic cases (median (range) age in months: 21.2 (14–26) versus 10 (3–17); p = 0.015). Seven tumors were histologically confirmed as low-grade astrocytomas. All patients received upfront chemotherapy and nutritional support. Although no patient died, all of them experienced tumor progression within 5.67 years since diagnosis and were treated with several lines of chemotherapy and/or surgery. Long-term sequelae included visual, pituitary and neurological dysfunction. Despite an excellent overall survival, PFS rates are poor in OPGs with DS. These patients invariably present visual, neurological or endocrine sequelae. Therefore, functional outcomes and quality-of-life measures should be considered in prospective trials involving patients with OPGs, aiming to identify “high-risk” patients and to better individualize treatment.
